A rare superficial pemphigus disease characterized by severe intractable pruritus with erythematous or urticarial plaques and sometimes vesicles organized in a herpetiform pattern. Mucosae are generally spared. Eosinophilia in peripheral blood and low titers of circulating autoantibodies are observed in many cases. Histology can show an aspect of either pemphigus (superficial or deep) or an intraepidermal infiltrate rich in eosinophils (eosinophilic spongiosis).
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version February 2024
Newly diagnosed with
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The Fairy Goddess Mother Project Inc.
The Fairy Goddess Mother Project is a 501(c)(3) Non-profit Rare Disease Patient Advocacy Organization dedicated to increasing awareness of the little-known, often misdiagnosed disease Vulvar Lichen Sclerosus (VLS), providing financial assistance to afflicted women in need of treatment, and supporting patients’ efforts to participate in clinical trials.
For a list of clinical trials in this disease area, please click here.